SAB1300282

Anti-GCS1 (C-term) antibody produced in rabbit

IgG fraction of antiserum, buffered aqueous solution

• Código UNSPSC: 12352203
• NACRES: NA.41

Propiedades

• origen biológico: rabbit
• Nivel de calidad: 100
• conjugado: unconjugated
• forma del anticuerpo: IgG fraction of antiserum
• tipo de anticuerpo: primary antibodies
• clon: polyclonal
• formulario: buffered aqueous solution
• reactividad de especies: mouse, human
• técnicas: indirect ELISA: 1:1000; western blot: 1:100-1:500
• Nº de acceso NCBI: NP_006293.2
• Nº de acceso UniProt: Q13724
• Condiciones de envío: dry ice
• temp. de almacenamiento: −20°C
• modificación del objetivo postraduccional: unmodified
• Información sobre el gen: human ... GCS1(7841)

Descripción

Descripción general

GCS1 cleaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor in a highly specific manner. Defects in GCS1 are the cause of type IIb congenital disorder of glycosylation (CDGIIb). This syndrome is also known as glucosidase I deficiency and is characterized by marked generalized hypotonia and hypomotility of the neonate, dysmorphic features, including a prominent occiput, short palpebral fissures, retrognathia, high arched palate, generalized edema, and hypoplastic genitalia. Symptoms include hepatomegaly, hypoventilation, feeding problems and seizures. The clinical course is progressive and survival is at most a few months.

Inmunógeno

GCS1 (GCS1_HUMAN, 801-836)
This antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide selected form the C-terminal region of human GCS1.

Forma física

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide.

Información de seguridad

• Código de clase de almacenamiento: 10 - Combustible liquids
• Clase de riesgo para el agua (WGK): nwg
• Punto de inflamabilidad (°F): Not applicable
• Punto de inflamabilidad (°C): Not applicable